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Risk factors for malignant ventricular arrhythmias in lamin A/C mutation carriers a European cohort study

Item Type:Article
Title:Risk factors for malignant ventricular arrhythmias in lamin A/C mutation carriers a European cohort study
Creators Name:van Rijsingen, I.A.W. and Arbustini, E. and Elliott, P.M. and Mogensen, J. and Hermans-van Ast, J.F. and van der Kooi, A.J. and van Tintelen, J.P. and van den Berg, M.P. and Pilotto, A. and Pasotti, M. and Jenkins, S. and Rowland, C. and Aslam, U. and Wilde, A.A.M. and Perrot, A. and Pankuweit, S. and Zwinderman, A.H. and Charron, P. and Pinto, Y.M.
Abstract:OBJECTIVES: The purpose of this study was to determine risk factors that predict malignant ventricular arrhythmias (MVA) in Lamin A/C (LMNA) mutation carriers. BACKGROUND: LMNA mutations cause a variety of clinical phenotypes, including dilated cardiomyopathy and conduction disease. Many LMNA mutation carriers have a poor prognosis, because of a high frequency of MVA and progression to end-stage heart failure. However, it is unclear how to identify mutation carriers that are at risk for MVA. METHODS: In this multicenter cohort of 269 LMNA mutation carriers, we evaluated risk factors for MVA, defined as sudden cardiac death, resuscitation, and appropriate implantable cardioverter-defibrillator (ICD) treatment. RESULTS: In a median follow-up period of 43 months (interquartile range: 17 to 101 months), 48 (18%) persons experienced a first episode of MVA: 11 persons received successful cardiopulmonary resuscitation, 25 received appropriate ICD treatment, and 12 persons died suddenly. Independent risk factors for MVA were nonsustained ventricular tachycardia, left ventricular ejection fraction <45% at the first clinical contact, male sex, and non-missense mutations (ins-del/truncating or mutations affecting splicing). MVA occurred only in persons with at least 2 of these risk factors. There was a cumulative risk for MVA per additional risk factor. CONCLUSIONS: Carriers of LMNA mutations with a high risk of MVA can be identified using these risk factors. This facilitates selection of LMNA mutation carriers who are most likely to benefit from an ICD.
Keywords:Cardiomyopathy, Implantable Cardioverter-Defibrillator, Lamin A/C, Risk Factors, Sudden Cardiac Death
Source:Journal of the American College of Cardiology
Page Range:493-500
Date:31 January 2012
Official Publication:https://doi.org/10.1016/j.jacc.2011.08.078
PubMed:View item in PubMed

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